Extramammary Paget's disease with diffuse involvement of the lower female genito-urinary system

Abstract

Extramammary Paget's disease of the lower female genito-urinary system is an uncommon neoplasm with a high rate of recurrence. A 52-year-old white female with a history of extramammary Paget's disease, originally excised in 1985 by skinning vulvectomy and who had multiple recurrences, including two in a vaginal graft and with extension to the urethra and the uterine cervix, in 1992 underwent an anterior pelvic exenteration for control of the disease process. Standard surgical management for extramammary Paget's disease without invasion or without an underlying adenocarcinoma is simple or skinning vulvectomy. However, because the disease commonly recurs, diffuse involvement may require more extensive surgery including pelvic exenteration in extraordinary cases.

Mammary and extramammary Paget's disease

“…20 42 Extramammary Paget's disease is often multifocal and in many cases it has been demonstrated histologically that the disease extends beyond the visible lesion-for example, primary vulval extramammary Paget's disease can spread into urethral, upper vaginal, and cervical epithelia. [43][44][45] Multiple surgical excisions may be required to control residual and recurrent disease. 3 4 20 Intraoperative frozen sectioning has been used to improve the rates of complete excision and improve prognosis.…”

Extramammary Paget's disease—a proliferation of adnexal origin?

“…EPD is a slowly progressing, intraepithelial, only occasionally invasive and extremely rarely metastasizing tumour, involving hair‐bearing skin of vulva and scrotum and the modified mucosa of the vulva and perineum. Extension to anal, urethral, vaginal and cervical mucosa has been observed 4,5 . The histogenesis of EPD is still elusive, but several hypotheses propose an origin from pluripotent epidermal stem cells, 4,6 intraepidermal ‘Toker cells’ 7,8 or apocrine glands 9 .…”

“…Extension to anal, urethral, vaginal and cervical mucosa has been observed. 4,5 The histogenesis of EPD is still elusive, but several hypotheses propose an origin from pluripotent epidermal stem cells, 4,6 intraepidermal 'Toker cells' 7,8 or apocrine glands. 9 Paget cells express mucin core proteins, 10 androgen receptors, 11 gross cystic disease fluid protein-15, 9 Her-2 neu [12][13][14] and intermediate filaments of the cytokeratin (CK) type 15 , particularly CK7 and CK20.…”

Paget Cells in Endometrial and Endocervical Curettings in a Patient With Recurrent Vulvar Paget’s Disease

“…Rare reports of Paget’s disease involving the genitourinary tract—lower tract with urethral involvement, 27 as well as upper tract involvement of the bladder 26 —demonstrate the potential for extensive spread. This extension to other pelvic organs makes endometrial involvement by Paget’s disease plausible.…”